Abstract

Background: Congenital Adrenal Hyperplasia (CAH) is the most common 46,XX DSDs, which occurred when one of the enzymes required for cortisol and aldosterone synthesis in adrenal gland is deficit, therefore performed adrenal hormone imbalance. Hormone replacement therapy using hydrocortisone is an option that allows control of the adrenal hormones balance. To monitor the effectiveness of therapy, measuring the adrenal hormones precursors (17-OHP and androstenedione) is required. Nevertheless, every therapy has side effect that must be controlled. The aim of this research is to seek 17-OHP and androstenedione levels post- compared to pre-therapy and also side effect of hydrocortisone therapy.
Methode: A pre-experimental study with one group pretest-posttest design was done to observe 17-OHP and androstenedione levels. The side effect post hydrocortisone therapy used a descriptive study. Samples of this research were all patients recorded in CEBIOR which are diagnosed as CAH after the clinical, cytogenetic and hormonal check-up and/or after the CYP21-gene mutation analysis then receive hydrocortisone therapy and follow-up. Blood plasma sample for hormonal pre-therapy and saliva sample for hormonal post therapy were analyzed using ELISA. This hormonal data was analyzed Wilcoxon test. Side effect data were presented in a descriptive narration.
Result: 38 cases (12.88%) of DSDs were registered in CEBIOR, and 23 were suitable to inclusion criteria. Wilcoxon analysis by comparing pre and post therapy hormonal data showed decline with p=0.987 for 17-OHP and elevation with p=0.04 for androsetedione. There were twelve subjects with short stature after hydrocortisone therapy and some of them complained intercurrent illness. The most common intercurrent illness was dehydration (44.44% of total complain).
Conclusion: Comparison between 17-OHP pre and post therapy is insignificantly decreased, whereas in androstenedione there is significant elevation. Side effects of hydrocortisone therapy are frequently caused by inadequate doses.
Keywords: congenital adrenal hyperplasia, CAH, hydrocortisone, 17-OHP, androstenedione, side effect